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Living with Dentin dysplasia type II

A rare, mild form of dentin dysplasia with normal tooth roots but abnormal baby teeth.

Understanding Dentin dysplasia type II

Dentin dysplasia type II is a rare, mild form of dentin dysplasia with normal tooth roots but abnormal baby (primary) teeth.

GARD lists features such as dentinogenesis imperfecta limited to baby teeth, thistle-tube-shaped pulp, and pulp stones. It is autosomal dominant.

Signs & symptoms

GARD lists these features:

  • Tooth changes limited to baby teeth
  • Thistle tube shaped pulp (inner tooth)
  • Pulp stones (calcium deposits in the tooth)

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include genetic specialists and gum, teeth and mouth specialists.

  3. Consider genetic testing

    Changes in DSPP are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.

Care & treatment

Care team
Genetic specialists (geneticists); Gum, teeth and mouth specialists (odontologists)
Expert care
Ask about multidisciplinary care centers for rare conditions
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with rare disease patient organizations for support
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
How will this affect baby teeth and adult teeth?
Can genetic testing of the DSPP gene help our family?
What dental care do we need?

Quick facts

InheritanceAutosomal Dominant
GenesDSPP

Changes in the DSPP gene cause this condition. It is autosomal dominant, so a changed copy of the gene from one parent can be enough.

Genetic

Also known as: anomalous dysplasia of dentin; anomalous dysplasia of dentine; coronal dentine dysplasia; dd-ii; dentin dysplasia, shields type ii; dentin dysplasia, type ii

Patient organizations

Rare-disease groups that can help

Source: NIH GARD · Dentin dysplasia type IIGARD ID 1806 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Dentin dysplasia type II.

Explore the Dentin dysplasia type II blog

Explore research for Dentin dysplasia type II

Explore actively recruiting Dentin dysplasia type II studies with HealthSpark.

HealthSpark

Find actively recruiting studies for Dentin dysplasia type II with HealthSpark. Share your city to explore research near you.

  • Recruiting studies from ClinicalTrials.gov
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  • Eligibility is confirmed by the study team

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Powered by HealthSpark, our AI chat helper · Study information from ClinicalTrials.gov · Eligibility is confirmed by the study team.

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